静脉内平滑肌瘤病2例并文献复习

Intravenous leiomyomatosis:two cases report and literature review

目的探讨静脉内平滑肌瘤病(IVL)的影像特征。方法回顾性分析2例经手术病理证实的IVL的临床表现及影像特征,并复习相关文献。结果MRI平扫显示子宫及附件区不规则肿块,T1WI呈低信号,T2WI呈稍高信号,子宫周围多发迂曲血管影;扩散加权成像(DWI)上病变扩散受限呈不均匀高信号;增强MRI显示病变明显强化,受累静脉不同程度增宽且强化欠均匀。对比增强磁共振血管成像显示盆腔多发不规则团片状明显不均匀强化灶,受累静脉管腔增宽,内见多发条索状充盈缺损。超声检查显示子宫角不均质回声,部分呈融合状,1例病灶向上与静脉血管相连,病灶内可见丰富的血流信号;另1例病灶内有少量血流信号。结论IVL影像表现具有一定特征,盆腔肿块伴邻近脉管增宽、管腔内充盈缺损是本病常见的影像征象。

Objective To investigate the radiological features of intravenous leiomyomatosis(IVL).Methods Clinical manifestations and imaging features of two patients with pathologically confirmed IVL were analyzed retrospectively,and relevant literatures were reviewed.Results MRI plain scan showed an irregular masse in the uterus and adnexal area with hypointensity on T1WI,slightly hyperintensity on T2WI.There were multiple tortuous vessels around uterus.DWI showed a limited diffusion and heterogeneous high signal intensity.The lesion was intensely enhanced after administration of contrast medium and the affected veins were widened in varying degrees and heterogenous enhancement.On contrast enhanced magnetic resonance angiography(CE-MRA),there were multiple irregular patches and obvious heterogeneous enhancement foci in the pelvis,the lumen of the involved veins were widened,and multiple cord-like filling defects were found.Ultrasound showed heterogeneous echo in the horn of uterus,part of which was fusion-like.In one case,veins were connected with the lesion,and abundant blood flow could be found;in the other case,there was a few signal of blood flow.Conclusions The imaging manifestations of IVL have certain characteristics.The common imaging signs of this disease are pelvic mass with widened adjacent vessels and intraluminal filling defect.